4
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Kartagener syndrome: A case report

      case-report

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Kartagener syndrome is a rare, autosomal recessive genetic disorder that causes defects in the action of ciliary movement, comprises of triad situs inversus, chronic sinusitis, and bronchiectasis. We present the case of a 3-year-old boy with repeated respiratory infections and pneumonic infections presenting with acute respiratory failure. He was diagnosed with Kartagener syndrome based on his clinical presentation and imaging features. The current diagnosis was consistent with severe acute bronchitis. He was managed initially with conventional medical therapy, but he didn’t respond and was transferred immediately to the pediatric intensive care unit where noninvasive ventilation was administered. He had shown significant predictors of early noninvasive ventilation failure and was mechanically ventilated, after which, he was disconnected from the ventilator and discharged without complications. In patients presenting with recurrent upper and lower respiratory tract infections, Kartagener syndrome should always be kept in mind. The correct diagnosis of this disorder in early life is very important to prevent complications and improve patients’ quality of life.

          Related collections

          Most cited references24

          • Record: found
          • Abstract: found
          • Article: not found

          Non-invasive ventilation in community-acquired pneumonia and severe acute respiratory failure.

          The use of non-invasive ventilation (NIV) in severe acute respiratory failure (ARF) due to community-acquired pneumonia (CAP) is controversial, and the risk factors for NIV failure in these patients are not well known. We assessed the characteristics and predictors of outcome of patients with CAP and severe ARF treated with NIV. We prospectively assessed 184 consecutive patients; 102 had "de novo" ARF, and 82 previous cardiac or respiratory disease. We defined successful NIV as avoidance of intubation and intensive care unit (ICU) survival at least 24 h in the ward. We assessed predictors of NIV failure and hospital mortality in multivariate analyses. Patients with "de novo" ARF failed NIV more frequently than patients with previous cardiac or respiratory disease (47, 46% versus 21, 26%, p = 0.007). Worsening radiologic infiltrate 24 h after admission, maximum Sepsis-Related Organ Failure Assessment (SOFA) score and, after 1 h of NIV, higher heart rate and lower PaO(2)/FiO(2) and bicarbonate independently predicted NIV failure. Likewise, maximum SOFA, NIV failure and older age independently predicted hospital mortality. Among intubated patients with "de novo" ARF, NIV duration was shorter in hospital survivors than non-survivors (32 ± 24 versus 78 ± 65 h, p = 0.014). In this group, longer duration of NIV before intubation was associated with decreased hospital survival (adjusted odds ratio 0.978, 95% confidence interval 0.962-0.995, p = 0.012). This association was not observed in patients with previous cardiac or respiratory disease. Successful NIV was strongly associated with better survival. If predictors for NIV failure are present, avoiding delayed intubation of patients with "de novo" ARF would potentially minimise mortality.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome.

            Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (dynein axonemal heavy chain 5) or intermediate(dynein axonemal intermediate chain 1) chain dynein genes in ciliary outer dynein arms, although a few mutations have been noted in other genes. Clinical molecular genetic testing for primary ciliary dyskinesia is available for the most common mutations. The respiratory manifestations of primary ciliary dyskinesia (chronic bronchitis leading to bronchiectasis, chronic rhino-sinusitis, and chronic otitis media)reflect impaired mucociliary clearance owing to defective axonemal structure. Ciliary ultrastructural analysis in most patients (>80%) reveals defective dynein arms, although defects in other axonemal components have also been observed. Approximately 50% of patients with primary ciliary dyskinesia have laterality defects (including situs inversus totalis and, less commonly, heterotaxy, and congenital heart disease),reflecting dysfunction of embryological nodal cilia. Male infertility is common and reflects defects in sperm tail axonemes. Most patients with primary ciliary dyskinesia have a history of neonatal respiratory distress, suggesting that motile cilia play a role in fluid clearance during the transition from a fetal to neonatal lung. Ciliopathies involving sensory cilia, including autosomal dominant or recessive polycystic kidney disease, Bardet-Biedl syndrome, and Alstrom syndrome, may have chronic respiratory symptoms and even bronchiectasis suggesting clinical overlap with primary ciliary dyskinesia.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Predictive factors for the success of noninvasive mask ventilation in infants and children with acute respiratory failure.

              Noninvasive mask ventilation (NIV) is a treatment option in acute respiratory failure in adults. This study was performed to determine prognostic variables for the success of NIV in a group of infants and children with respiratory failure for a wide range of reasons. Prospective, clinical study. Multidisciplinary, neonatal-pediatric intensive care unit of a university teaching hospital. Descriptive study of infants and children 80% after 1 hr of NIV predicted nonresponse with a sensitivity of 56%, specificity of 83%, and positive and negative predictive value of 71%. NIV can be successfully applied to infants and children with acute respiratory failure in the setting of a pediatric intensive care unit. The level of Fio2 after 1 hr of NIV may be a predictive factor for the treatment success.
                Bookmark

                Author and article information

                Journal
                Can J Respir Ther
                Can J Respir Ther
                CJRT
                Canadian Journal of Respiratory Therapy: CJRT = Revue Canadienne de la Thérapie Respiratoire : RCTR
                Canadian Society of Respiratory Therapists
                1205-9838
                2368-6820
                21 April 2021
                2021
                : 57
                : 44-48
                Affiliations
                Pediatric Intensive Care Unit, Children’s Damascus University Hospital, Damascus, Syria
                Author notes
                Correspondence: Rahaf Ibrahim, Damascus, Syria. Tel: 00963934518980; E-mail: Dr.r.e345@ 123456gmail.com
                Article
                064
                10.29390/cjrt-2020-064
                8059757
                33912655
                075c68bc-fb8d-4c76-8dda-3179e7c3fbc7

                This open-access article is distributed under the terms of the Creative Commons Attribution Non-Commercial License (CC BY-NC) ( http://creativecommons.org/licenses/by-nc/4.0/), which permits reuse, distribution and reproduction of the article, provided that the original work is properly cited and the reuse is restricted to noncommercial purposes. For commercial reuse, contact editor@ 123456csrt.com

                History
                Categories
                Case Report

                kartagener syndrome,primary ciliary dyskinesia,situs inversus,bronchiectasis,dextrocardia,non-invasive ventilation

                Comments

                Comment on this article